单孔腹腔镜Deloyers术在先天性长段型巨结肠一期根治术中的应用

目的总结单孔腹腔镜Deloyers术在先天性长段型巨结肠手术中的应用经验及技巧。方法回顾性分析2018年1月至2021年5月广州市妇女儿童医疗中心胃肠外科单孔腹腔镜Deloyers术治疗的16例先天性长段型巨结肠患儿的临床资料, 其中男11例, 女5例;年龄为3～10个月;体重为5.0～12.8 kg;均行升结肠倒转, 评价其手术效果, 总结单孔腹腔镜下Deloyers术的经验。结果 16例患儿均一期完成单孔腹腔镜下巨结肠根治术(Deloyers倒转法), 术中完成单孔腹腔镜下结肠韧带松解及乙状结肠、直肠游离后, 将结肠经脐部提出腹腔外直视下完成结肠系膜分离及升结肠倒转, 无中转开放或需增加Trocar病例。手术时间为(217±30)min, 出血量为5(2～10)ml, 术后住院时间为7(5～8)d。术后均恢复良好, 随访6～46个月, 无出血、肠梗阻、吻合口漏、便秘复发、大便失禁等并发症, 术后6个月后患儿排便次数为2～5次/d, 术后腹部均无可视性瘢痕。结论单孔腹腔镜Deloyers术可应用于先天性长段型巨结肠的一期根治术, 具有操作精准、便捷、创伤小、美观等优点, 术后短期效果...     

经肛门拖出及辅助腹部小切口根治长段型巨结肠33例报告

目的探讨经肛门拖出及/或选择性辅加腹部小切口根治长段型巨结肠的可行性与疗效。方法2001年1月～2005年3月经用该术式治疗婴儿长段型巨结肠33例。其中病变段位于乙状结肠上段16例,降结肠11例,脾曲4例,横结肠右侧2例。全组病例均经钡剂灌肠、肛门直肠测压、手术及病理检查确诊。实施I期手术32例,II期手术1例。结果17例单独经肛门拖出结肠,16例辅加腹部3～5cm小切口协助完成手术。切除病变结肠平均长度为39.2cm(32～63cm)。平均手术时间为145min(110～190min)。出血约10～50ml。术后恢复良好,6例出现肛周轻度红肿、糜烂,均于术后6～11d痊愈出院。30例经3个月～4.5年随访,生长发育良好,3个月内每日排便4～8次,6个月后每日2～3次。5例发生结肠炎,经保守治疗痊愈。轻度污粪及便秘各1例。无腹部并发症及吻合口狭窄。结论经肛门结肠拖出术根治婴儿长段型巨结肠方法可行,且安全、有效,操作较简便。对病变位于降结肠以上、结肠系膜较短者,可辅加腹部小切口协助完成手术,值得推荐。     

经脐腹腔镜结肠拖出术治疗先天性巨结肠症

目的 介绍经脐腹腔镜下拖出术治疗先天性巨结肠的手术方法以及临床手术经验.方法 回顾2009年6月至9月,对9例先天性巨结肠患儿采用经脐腹腔镜下拖出术进行治疗.患儿平均年龄为31.9个月(年龄范围在1～99个月),平均体重为16.2 kg(体重范围在4.7～25 kg).患儿脐窝处分别置入3个5 mm trocar,在腹腔镜镜头监控下,使用特制弯曲手柄型腹腔镜操作杆分离相应肠系膜及血管.扩肛,分离直肠肌肉与黏膜,然后将病变肠管呈袖套式拖出肛门外切除,行结肠肛门心形吻合术.记录术前各项检查以及手术相关数据.对手术患儿进行随访,记录术后并发症的发生情况.结果 平均手术时间为155 min(时间范围在110～185 min),平均失血量为40 ml(失血范围在10～100 ml).没有患儿术中转为传统腹腔镜手术或者开腹手术.无一例患儿术中出现腹部血管、肠管、输尿管、输精管损伤.患儿术后3 d进流食,术后7 d可出院.在随访期间,详细记录患儿术后并发症和恢复情况:1例患儿术后16 d出现小肠结肠炎,经过抗感染等对症治疗后好转;其余患儿均未出现术后并发症(吻合口狭窄、吻合口漏、便秘、污粪、大便失禁、腹泻、小肠结肠炎).术毕脐窝稍红肿,术后30d患儿复诊未见明显手术瘢痕.结论 经脐腹腔镜拖出术来治疗先天性巨结肠具有简单易行、美观的优点,适用于长段型巨结肠患儿.     

微创切口在治疗先天性巨结肠中的应用

目的介绍微创切口手术治疗先天性巨结肠的经验,着重比较不同手术切口的美容效果。方法回顾性分析1990年7月～2004年12月收治的先天性巨结肠患者的临床资料,按手术方式不同分为两组：A组为传统腹会阴根治组（n=102,为1990年7月～1998年12月病例）;B组为微创切口组（n=224,为1999年1月～2004年12月病例）。重点分析手术方式与手术创伤指标（手术时间、出血量、肠功能恢复时间）、术后短期并发症、腹壁切口美容以及排便功能控制的关系。结果两组患者的性别、病变分类无显著性差异。B组平均年龄小、手术创伤指标明显优于A组。A组术后并发,腹部切口感染14例,隔膜形成9例（经再次上夹治愈）,残留腹部切口长12～16cm。B组无相关并发症,腹部仅有0.5cm切口或4～5cm绕脐切口。两组排便控制功能优良,差异无统计学意义。结论合理选择、组合经肛门巨结肠根治＋腹腔镜辅助或绕脐切口3种手术是安全有效的微创手术方式,创伤小,恢复快,并发症少,美容效果佳,近期排便控制功能优良。     

腹腔镜辅助与开腹手术治疗先天性巨结肠的 Meta 分析

目的：通过 Meta 分析腹腔镜辅助与开腹手术治疗先天性巨结肠的疗效与安全性,评价腹腔镜辅助一期手术根治先天性巨结肠的优势。方法检索1998年1月至2013年8月 Pubmed、西文生物医学期刊文献数据库、CCTR、CNKI、中国生物医学文献数据库发表的相关腹腔镜辅助与传统开腹手术治疗先天性巨结肠疗效比较的文献,比较手术时间、术中出血量、术后住院时间、胃肠功能恢复时间、术后早期并发症的发生率（切口感染、粘连性肠梗阻、尿潴留、术后出血）以及远期并发症的发生率（小肠结肠炎、大便失禁、便秘、污粪）。用 Review Manger 5．2软件进行 Meta 分析。结果共9篇文献入选,9篇文献记录先天性巨结肠手术患儿456例,其中腹腔镜手术239例,开腹手术195例。Meta 分析结果显示：腹腔镜手术较开腹手术出血少,住院时间短,差异有统计学意义（P 值均≤0．05）,手术时间和术后胃肠功能恢复时间比较,差异无统计学意义（P 值均≥0．05）,术后早期并发症的发生率分别为11．61％、21．53％,差异有统计学意义（P 值≤0．05）,术后远期并发症率分别为32．90％、42．56％,差异无统计学意义（P 值≥0．05）。结论腹腔镜辅助一期手术根治先天性巨结肠创伤小,恢复快,能有效减少术后早期并发症的发生。     

腹腔镜辅助治疗长段型巨结肠的临床应用

腹腔镜辅助治疗长段型巨结肠可使长段型巨结肠患儿获得彻底的早期治疗,充分发挥了腹腔镜的微创手术优点,损伤小、恢复快、腹腔干扰小,且美容效果好,腹腔镜在长段型巨结肠根治术中的作用是经肛门巨结肠根治术及开腹手术所无法替代的.     

腹腔镜与开腹手术治疗新生儿长段型巨结肠的对比研究

目的 探讨腹腔镜与开腹手术治疗新生儿长段型巨结肠的近期疗效.方法 2007年1月至2009年1月作者收治新生儿长段型巨结肠17例,均表现为顽固性便秘,钡灌肠24 h拍片均见大量钡剂残留.8例采取腹腔镜手术,其中7例直肠肛管测压无松弛反射,直肠黏膜乙酰胆碱酯酶染色均为阳性;9例采取开腹手术,7例无松弛反射,直肠黏膜乙酰胆碱酯酶染色均为阳性.结果 17例中,5例行左半结肠切除术,12例行右半结肠切除术.均治愈,并发切口裂开1例,吻合口瘘1例,为开腹手术患儿.随访左半结肠切除患儿术后大便形状正常;结肠次全切除患儿术后1个月左有出现腹泻,后逐渐好转;随访期间3例发生小肠结肠炎,经抗炎、洗肠等治疗后痊愈,其中1例为腹腔镜手术,2例为开腹手术.结论 腹腔镜巨结肠根治术治疗新生儿长段型巨结肠可取得满意的近期疗效.     

单孔腹腔镜辅助经脐“Z”形切口治疗消化道出血

目的探讨单孔腹腔镜辅助经脐"Z"形切口治疗小儿消化道出血的可行性。方法采用单孔腹腔镜辅助经脐孔"Z"形切口对25例消化道出血患儿进行治疗。于脐孔置入10 mm Trocar,进无损伤操作钳,探查找到憩室或重复畸形肠段后,延长脐部切口成"Z"形,将病变肠管提出腹腔外行肠切除肠吻合术。1例术中发现并右侧腹股沟隐性斜疝,行疝囊高位结扎。结果 25例手术获得成功,无中转开腹手术。手术时间65～95 min,平均78 min。术后病理检查证实为梅克尔憩室22例,小肠重复畸形3例,18例有胃黏膜异位。术后4 d进流质饮食,5～8 d出院。无脐疝、肠瘘及肠梗阻等并发症发生。结论单孔腹腔镜辅助经脐"Z"形切口治疗因梅克尔憩室或肠重复畸形引起的消化道出血安全可行,具有创伤小、切口隐蔽、外形美观等优点。     

腹腔镜辅助下先天性巨结肠升结肠拖出根治术

目的 运用腹腔镜及超声刀技术对不能用经肛门拖出术式的小儿长段型巨结肠病例施行根治手术，充分发挥腹腔镜的微创手术优点，探讨其操作方法和特点。方法 对2000年6月至2001年11月收治的8例长段型先天性巨结肠患儿，使用腹腔镜及超声刀技术施行升结肠逆时针翻转拖出（Deloyers法）的先天性巨结肠根治术（改良Soave术）。观察手术过程、术中出血、术后合并症的出现、大便排出等情况。术后门诊随访。结果 8例手术均获成功，手术时间190－240min，平均218min。手术出血最多1例为20ml，余均少于5ml，无术后继发性出血，无术中和术后早期并发症。8例术后均在门诊复诊，随访3－20个月，所有患儿每日排便1－8次，无大便失禁或污粪。5例术后3个月大便稀糊状，半年后转成形大便。结论 应用腹腔镜超声刀技术可有效地施行升结肠拖出手术治疗长段型巨结肠，手术可弥补经肛门拖出手术和开腹手术的不足，近期效果良好。     

经肛门I期巨结肠根治术方法改进探讨

目的研究经肛门I期巨结肠根治手术并发症的预防措施,探索改进手术方法,提高其治疗效果。方法10例先天性巨结肠及2例继发性巨结肠经肛门I期根治手术,采用粘膜、环肌、纵肌梯度分离推进,从直肠侧方腹膜返折处先进入盆腔,经手术中和术后观察及3～18月(平均13.2月)随诊综合分析。结果10例经肛门I期根治的先天性巨结肠、2例继发性巨结肠病儿,手术时间缩短,术后反应轻。肛门无狭窄,排便每日1～2次,无污粪发生。结论经肛门I期巨结肠根治手术,采用粘膜、环肌、纵肌梯度分离推进式进入,从直肠侧方腹膜返折处先进入盆腔,可避免尿道及其邻近器官损伤,达到与经腹切口或腹腔镜下巨结肠根治术同样的手术效果。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

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孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.